[Two clinical cases of sporadic lymphangioleiomyomatosis]. / Linfangioleiomiomatosis esporádica. Dos casos clínicos.
Rev Med Inst Mex Seguro Soc
; 50(3): 319-25, 2012.
Article
em Es
| MEDLINE
| ID: mdl-23182263
Lymphangioleiomyomatosis (LAM) is a rare lung disease of unknown etiology, described since 1918 associated with tuberous sclerosis complex (TSC-LAM) and are reported sporadically (S-LAM). It is classified within the group of interstitial lung diseases (ILD) and currently the European Respiratory Society (ERS) has published guidelines for diagnosis and treatment. The objective is to evaluate the clinical presentation of two patients, evolution, management, and review of current treatment. Both patients admitted in our hospital for examination, presenting with spontaneous pneumothorax. Their CT scan shows characteristic cystic lesions and thoracotomy with lung biopsy was performed because lack of expansion and to confirming the diagnosis. Despite the controversy about the optimal management of these patients, there is evidence about the use of progesterone routinely in patients with rapid deterioration of respiratory function when it was provided for a period of at least 12 months. Due to the rareness of the disease, it requires a patient registry to evaluate the use of experimental drugs or include them in research protocols to improve their prognosis.
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Coleções:
01-internacional
Base de dados:
MEDLINE
Assunto principal:
Linfangioleiomiomatose
Tipo de estudo:
Guideline
/
Prognostic_studies
Limite:
Adult
/
Female
/
Humans
Idioma:
Es
Revista:
Rev Med Inst Mex Seguro Soc
Assunto da revista:
MEDICINA
/
MEDICINA SOCIAL
Ano de publicação:
2012
Tipo de documento:
Article
País de publicação:
México